Recombinant人HIBCH protein (ab124585)

概述

描述

  • 性质Recombinant
  • 来源Escherichia coli
  • 氨基酸序列
    • AccessionQ6NVY1
    • 种属Human
    • 序列MGSSHHHHHH SSGLVPRGSH MGSHMDAAEE VLLEKKGCTG VITLNRPKFL NALTLNMIRQ IYPQLKKWEQ DPETFLIIIK GAGGKAFCAG GDIRVISEAE KAKQKIAPVF FREEYMLNNA VGSCQKPYVA LIHGITMGGG VGLSVHGQFR VATEKCLFAM PETAIGLFPD VGGGYFLPRL QGKLGYFLAL TGFRLKGRDV YRAGIATHFV DSEKLAMLEE DLLALKSPSK ENIASVLENY HTESKIDRDK SFILEEHMDK INSCFSANTV EEIIENLQQD GSSFALEQLK VINKMSPTSL KITLRQLMEG SSKTLQEVLT MEYRLSQACM RGHDFHEGVR AVLIDKDQSP KWKPADLKEV TEEDLNNHFK SLGSSDLKF
    • 分子量42 kDa including tags
    • 氨基酸33 to 386
    • 标签His tag N-Terminus

技术指标

Our Abpromise guarantee covers the use of ab124585 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

  • 应用

    SDS-PAGE

    Mass Spectrometry

  • 质谱法
    MALDI-TOF
  • 纯度> 90 % SDS-PAGE.
    ab124585 is purified using conventional chromatography techniques.
  • 形式Liquid
  • Concentration information loading...

制备和贮存

  • 稳定性和存储

    Shipped at 4°C. Upon delivery aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.

    pH: 8.00
    Constituents: 0.02% DTT, 0.32% Tris HCl, 10% Glycerol, 1.17% Sodium chloride

常规信息

  • 别名
    • 3 hydroxyisobutyryl Coenzyme A hydrolase
    • 3 hydroxyisobutyryl Coenzyme A hydrolase, mitochondrial
    • 3-hydroxyisobutyryl-CoA hydrolase
    • 3-hydroxyisobutyryl-coenzyme A hydrolase
    • BETA HYDROXYISOBUTYRYL COENZYME A HYDROLASE
    • HIB CoA hydrolase
    • HIB-CoA hydrolase
    • HIBCH
    • HIBCH_HUMAN
    • HIBCoA hydrolase
    • HIBYL CoA H
    • HIBYL CoAH
    • HIBYL-CoA-H
    • HIBYLCoA H
    • HIBYLCoAH
    • mitochondrial
    see all
  • 功能Hydrolyzes 3-hydroxyisobutyryl-CoA (HIBYL-CoA), a saline catabolite. Has high activity toward isobutyryl-CoA. Could be an isobutyryl-CoA dehydrogenase that functions in valine catabolism. Also hydrolyzes 3-hydroxypropanoyl-CoA.
  • 组织特异性Highly expressed in liver and kidney, also detected in heart, muscle and brain (at protein level). Not detected in lung.
  • 通路Amino-acid degradation; L-valine degradation.
  • 疾病相关Defects in HIBCH are the cause of HIBCH deficiency (HIBCHD) [MIM:250620]; also known as deficiency of beta-hydroxyisobutyryl CoA deacylase or methacrylic aciduria. The enzyme defect results in accumulation of methacrylyl-CoA, a highly reactive compound, which readily undergoes addition reactions with free sulfhydryl groups. Affected individuals showed delayed development of motor skills, hypotonia, initial poor feeding, and a deterioration in neurological function during first stages of life.
  • 序列相似性Belongs to the enoyl-CoA hydratase/isomerase family.
  • 细胞定位Mitochondrion.
  • Information by UniProt

Recombinant Human HIBCH protein 图像

  • 15% SDS-PAGE showing ab124585 at approximately 42.1kDa (3µg).

Recombinant Human HIBCH protein (ab124585)参考文献

ab124585 has not yet been referenced specifically in any publications.

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